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Blood, 15 May 2008, Vol. 111, No. 10, pp. 4973-4978. Prepublished online as a Blood First Edition Paper on March 14, 2008; DOI 10.1182/blood-2007-12-126391. This Article Full Text Full Text (PDF) All Versions of this Article: blood-2007-12-126391v1 111/10/4973    most recent Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Rights and Permissions Citing Articles Citing Articles via CrossRef Google Scholar Articles by Zhang, J. Articles by Broze, G. J. PubMed PubMed Citation Articles by Zhang, J. Articles by Broze, G. J., Jr Related Collections Hemostasis, Thrombosis, and Vascular Biology Social Bookmarking                   What's this?  Previous Article  |  Table of Contents  |  Next Article  HEMOSTASIS, THROMBOSIS, AND VASCULAR BIOLOGY Protein Z–dependent protease inhibitor deficiency produces a more severe murine phenotype than protein Z deficiency Jing Zhang1,*, Yizheng Tu1,*, Lan Lu1, Nina Lasky1, and George J. Broze, Jr1 1 Division of Hematology, Washington University, St Louis, MO Protein Z (PZ) is a plasma vitamin K–dependent protein that functions as a cofactor to dramatically enhance the inhibition of coagulation factor Xa by the serpin, protein Z–dependent protease inhibitor (ZPI). In vitro, ZPI not only inhibits factor Xa in a calcium ion–, phospholipid-, and PZ-dependent fashion, but also directly inhibits coagulation factor XIa. In murine gene-deletion models, PZ and ZPI deficiency enhances thrombosis following arterial injury and increases mortality from pulmonary thromboembolism following collagen/epinephrine infusion. On a factor VLeiden genetic background, ZPI deficiency produces a significantly more severe phenotype than PZ deficiency, implying that factor XIa inhibition by ZPI is physiologically relevant. The studies in mice suggest that human PZ and ZPI deficiency would be associated with a modest thrombotic risk with ZPI deficiency producing a more severe phenotype. CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?

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